|Other names||Ménière's syndrome, idiopathic endolymphatic hydrops|
|Diagram of the inner ear|
|Symptoms||Feeling like the world is spinning, ringing in the ears, hearing loss, fullness in the ear|
|Duration||20 minutes to few hours per episode|
|Risk factors||Family history|
|Diagnostic method||Based on symptoms, hearing test|
|Differential diagnosis||Vestibular migraine, transient ischemic attack|
|Treatment||Low salt diet, diuretics, corticosteroids, counselling|
|Prognosis||After ~10 years hearing loss and chronic ringing|
|Frequency||0.3–1.9 per 1,000|
Ménière's disease (MD) is a disorder of the inner ear that is characterized by episodes of feeling like the world is spinning (vertigo), ringing in the ears (tinnitus), hearing loss, and a fullness in the ear.Typically, only one ear is affected initially; however, over time both ears may become involved. Episodes generally last from 20 minutes to a few hours. The time between episodes varies. The hearing loss and ringing in the ears can become constant over time.
The inner ear is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the temporal bone of the skull with a system of passages comprising two main functional parts:
Vertigo is a symptom where a person feels as if they or the objects around them are moving when they are not. Often it feels like a spinning or swaying movement. This may be associated with nausea, vomiting, sweating, or difficulties walking. It is typically worse when the head is moved. Vertigo is the most common type of dizziness.
Tinnitus is the hearing of sound when no external sound is present. While often described as a ringing, it may also sound like a clicking, hiss or roaring. Rarely, unclear voices or music are heard. The sound may be soft or loud, low or high pitched, and present in one or both ears. Most of the time, it comes on gradually. In some people, the sound causes depression or anxiety and can interfere with concentration.
The cause of Ménière's disease is unclear but likely involves both genetic and environmental factors.A number of theories exist for why it occurs including constrictions in blood vessels, viral infections, and autoimmune reactions. About 10% of cases run in families. Symptoms are believed to occur as the result of increased fluid build up in the labyrinth of the inner ear. Diagnosis is based on the symptoms and, frequently, a hearing test. Other conditions that may produce similar symptoms include vestibular migraine and transient ischemic attack.
The membranous labyrinth is a collection of fluid filled tubes and chambers which contain the receptors for the senses of equilibrium and hearing. It is lodged within the bony labyrinth in the inner ear and has the same general form; it is, however, considerably smaller and is partly separated from the bony walls by a quantity of fluid, the perilymph.
A hearing test provides an evaluation of the sensitivity of a person's sense of hearing and is most often performed by an audiologist using an audiometer. An audiometer is used to determine a person's hearing sensitivity at different frequencies. There are other hearing tests as well, e.g., Weber test and Rinne test.
A transient ischemic attack (TIA) is a brief episode of neurological dysfunction caused by loss of blood flow (ischemia) in the brain, spinal cord, or retina, without tissue death (infarction). TIAs have the same underlying mechanism as ischemic strokes. Both are caused by a disruption in blood flow to the brain, or cerebral blood flow (CBF). The definition of TIA was classically based on duration of neurological symptoms. The current widely-accepted definition is called "tissue-based" because it is based on imaging, not time. The American Heart Association and the American Stroke Association (AHA/ASA) now define TIA as a brief episode of neurological dysfunction with a vascular cause, with clinical symptoms typically lasting less than one hour, and without evidence of infarction on imaging.
A cure does not exist.Attacks are often treated with medications to help with the nausea and anxiety. Measures to prevent attacks are overall poorly supported by the evidence. A low salt diet, diuretics, and corticosteroids may be tried. Physical therapy may help with balance and counselling may help with anxiety. Injections into the ear or surgery may also be tried if other measures are not effective but are associated with risks. The use of tympanostomy tubes, while popular, is not supported.
Nausea is an unpleasant, diffuse sensation of unease and discomfort, often perceived as an urge to vomit. While not painful, it can be a debilitating symptom if prolonged, and has been described as placing discomfort on the chest, upper abdomen, or back of the throat.
Anxiety is an emotion characterized by an unpleasant state of inner turmoil, often accompanied by nervous behaviour such as pacing back and forth, somatic complaints, and rumination. It is the subjectively unpleasant feelings of dread over anticipated events, such as the feeling of imminent death.
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involved in a wide range of physiological processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior.
Ménière's disease was first identified in the early 1800s by Prosper Ménière.It affects between 0.3 and 1.9 per 1,000 people. It most often starts in people 40 to 60 years old. Females are more commonly affected than males. After 5 to 15 years of symptoms, the episodes of the world spinning generally stop and the person is left with mild loss of balance, moderately poor hearing in the affected ear, and ringing in their ear.
Ménière's is characterized by recurrent episodes of vertigo, hearing loss and tinnitus; episodes may be accompanied by a headache and a feeling of fullness in the ears.
People may also experience additional symptoms related to irregular reactions of the autonomic nervous system. These symptoms are not symptoms of Meniere's disease per se, but rather are side effects resulting from failure of the organ of hearing and balance, and include nausea, vomiting, and sweating—which are typically symptoms of vertigo, and not of Ménière's.This includes a sensation of being pushed sharply to the floor from behind.
The autonomic nervous system (ANS), formerly the vegetative nervous system, is a division of the peripheral nervous system that supplies smooth muscle and glands, and thus influences the function of internal organs. The autonomic nervous system is a control system that acts largely unconsciously and regulates bodily functions such as the heart rate, digestion, respiratory rate, pupillary response, urination, and sexual arousal. This system is the primary mechanism in control of the fight-or-flight response.
Vomiting is the involuntary, forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose.
Sudden falls without loss of consciousness (drop attacks) may be experienced by some people.
The cause of Ménière's disease is unclear but likely involves both genetic and environmental factors.A number of theories exist including constrictions in blood vessels, viral infections, autoimmune reactions.
The initial triggers of Ménière's disease are not fully understood, with a variety of potential inflammatory causes that lead to endolymphatic hydrops (EH), a distension of the endolymphatic spaces in the inner ear. EH, in turn, is strongly associated with developing MD,but not everyone with EH develops MD: "The relationship between endolymphatic hydrops and Meniere's disease is not a simple, ideal correlation."
Additionally, in fully developed MD the balance system (vestibular system) and the hearing system (cochlea) of the inner ear are affected, but there are cases where EH affects only one of the two systems strong enough to cause symptoms. The corresponding subtypes of MD are called vestibular MD, showing symptoms of vertigo, and cochlear MD, showing symptoms of hearing loss and tinnitus.
The mechanism of MD is not fully explained by EH, but fully developed EH may mechanically and chemically interfere with the sensory cells for balance and hearing, which can lead to temporary dysfunction and even to death of the sensory cells, which in turn can cause the typical symptoms of MD: vertigo, hearing loss, and tinnitus.
The diagnostic criteria as of 2015 define definite MD and probable MD as follows:
- Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
- Audiometrically documented low- to medium-frequency sensorineural hearing loss in the affected ear on at least 1 occasion before, during, or after one of the episodes of vertigo
- Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
- Not better accounted for by another vestibular diagnosis
- Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
- Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the reported ear
- Not better accounted for by another vestibular diagnosis
A common and important symptom of MD is hypersensitivity to sounds.This hypersensitivity is easily diagnosed by measuring the loudness discomfort levels (LDLs).
Symptoms of MD overlap with migraine-associated vertigo (MAV) in many ways, but when hearing loss develops in MAV is usually in both ears, and this is rare in MD, and hearing loss generally does not progress in MAV as it does in MD.
People who have had a transient ischemic attack (TIA) or stroke can present with symptoms similar to MD, and in people at risk magnetic resonance imaging (MRI) should be conducted to exclude TIA or stroke.
Other vestibular conditions that should be excluded include vestibular paroxysmia, recurrent unilateral vestibulopathy, vestibular schwannoma, or a tumor of the endolymphatic sac.
There is no cure for Ménière's disease but medications, diet, physical therapy and counseling, and some surgical approaches can be used to manage it.
During MD episodes, medications to reduce nausea are used, as are drugs to reduce the anxiety caused by vertigo.
For longer-term treatment to stop progression, the evidence base is weak for all treatments.
Although a causal relation between allergy and Ménière's disease is uncertain, medication to control allergies may be helpful.
Diuretics, such as the thiazide-like diuretic chlortalidone, are widely used to manage Ménière's on the theory that it reduces fluid buildup in the ear. Based on evidence from multiple but small clinical trials, diuretics appear to be useful for reducing the frequency of episodes of dizziness but do not seem to prevent hearing loss.
In cases where there is hearing loss and continuing severe episodes of vertigo, a chemical labyrinthectomy, in which a medication such as gentamicin is injected into the middle ear and kills parts of the vestibular apparatus.This treatment has the risk of worsening hearing loss.
People with MD are often advised to reduce their salt intake.Reducing salt intake, however, has not been well studied. Based on the assumption that MD is similar in nature to a migraine, some advise eliminating "migraine triggers" like caffeine. However, the evidence for this is weak. There is no high quality evidence that changing diet by restricting salt, caffeine or alcohol improves symptoms.
While use of physical therapy early after the onset of MD is probably not useful due to the fluctuating disease course, physical therapy to help retraining of the balance system appears to be useful to reduce both subjective and objective deficits in balance over the longer term.
The psychological distress caused by the vertigo and hearing loss may worsen the condition in some people.Counseling may be useful to manage the distress, as may education and relaxation techniques.
If symptoms do not improve with typical treatment, surgery may be considered.Surgery to decompress the endolymphatic sac is one option. A systematic review in 2015 found that three methods of decompression have been used: simple decompression, insertion of a shunt; and removal of the sac. It found some evidence that all three methods were useful for reducing dizziness, but that the level of evidence was low, as trials were not blinded nor were there placebo controls.
Another 2015 review found that shunts used in these surgeries often turn out to be displaced or misplaced in autopsies, and recommended their use only in cases where the condition is uncontrolled and affecting both ears.A systematic review from 2014 found that in at least 75% of people EL sac decompression was effective at controlling vertigo in the short term (>1 year of follow-up) and long term (>24 months).
It has been estimated that about 30% of people with Meniere's disease have eustachian tube dysfunction.While a 2005 review found tentative evidence of benefit from tympanostomy tubes for improvement in the unsteadiness associated with the disease, a 2014 review concluded that they are not supported.
Destructive surgeries are irreversible and involve removing entire functionality of most, if not all, of the affected ear; as of 2013, there was almost no evidence with which to judge whether these surgeries are effective.The inner ear itself can be surgically removed via labyrinthectomy, although hearing is always completely lost in the affected ear with this operation. The surgeon can also cut the nerve to the balance portion of the inner ear in a vestibular neurectomy. The hearing is often mostly preserved; however, the surgery involves cutting open into the lining of the brain, and a hospital stay of a few days for monitoring would be required.
Ménière's disease usually starts confined to one ear; it appears that it extends to both ears in about 30% of cases.
People may start out with only one symptom, but in MD all three appear with time.Hearing loss usually fluctuates in the beginning stages and becomes more permanent in later stages. MD has a course of 5–15 years, and people generally end up with mild disequilibrium, tinnitus, and moderate hearing loss in one ear.
From 3% to 11% of diagnosed dizziness in neuro-otological clinics are due to Meniere's.The annual incidence rate is estimated to be about 15/100,000 and the prevalence rate is about 218/100,000, and around 15% of people with Meniere's disease are older than 65. In around 9% of cases a relative also had MD, signalling that there may be a genetic predisposition in some cases.
The odds of MD are greater for people of white ethnicity, with severe obesity, and women.Several conditions are often comorbid with MD, including arthritis, psoriasis, gastroesophageal reflux disease, irritable bowel syndrome, and migraine.
The condition is named after the French physician Prosper Ménière, who in an 1861 article described the main symptoms and was the first to suggest a single disorder for all of the symptoms, in the combined organ of balance and hearing in the inner ear.
The American Academy of Otolaryngology–Head and Neck Surgery Committee on Hearing and Equilibrium (AAO HNS CHE) set criteria for diagnosing Ménière's, as well as defining two sub-categories of Ménière's: cochlear (without vertigo) and vestibular (without deafness).
In 1972, the Academy defined criteria for diagnosing Ménière's disease as:
In 1985, this list changed to alter wording, such as changing "deafness" to "hearing loss associated with tinnitus, characteristically of low frequencies" and requiring more than one attack of vertigo to diagnose.Finally in 1995, the list was again altered to allow for degrees of the disease:
In 2015, the International Classification for Vestibular Disorders Committee of the Barany Society published consensus diagnostic criteria in collaboration with the American Academy of Otolaryngology – Head and Neck Surgery, the European Academy of Otology & Neuro-Otology, the Japan Society for Equilibrium Research, and the Korean Balance Society.
Prosper Menière was a French doctor who first identified that the inner ear could be the source of a condition combining vertigo, hearing loss and tinnitus, which is now known as Ménière's disease.
Otitis media is a group of inflammatory diseases of the middle ear. The two main types are acute otitis media (AOM) and otitis media with effusion (OME). AOM is an infection of rapid onset that usually presents with ear pain. In young children this may result in pulling at the ear, increased crying, and poor sleep. Decreased eating and a fever may also be present. OME is typically not associated with symptoms. Occasionally a feeling of fullness is described. It is defined as the presence of non-infectious fluid in the middle ear for more than three months. Chronic suppurative otitis media (CSOM) is middle ear inflammation that results in discharge from the ear for more than three months. It may be a complication of acute otitis media. Pain is rarely present. All three types of otitis media may be associated with hearing loss. The hearing loss in OME, due to its chronic nature, may affect a child's ability to learn.
Ototoxicity is the property of being toxic to the ear (oto-), specifically the cochlea or auditory nerve and sometimes the vestibular system, for example, as a side effect of a drug. The effects of ototoxicity can be reversible and temporary, or irreversible and permanent. It has been recognized since the 19th century. There are many well-known ototoxic drugs used in clinical situations, and they are prescribed, despite the risk of hearing disorders, to very serious health conditions. Ototoxic drugs include antibiotics such as gentamicin, streptomycin, tobramycin, loop diuretics such as furosemide and platinum-based chemotherapy agents such as cisplatin, carboplatin, and vincristine. A number of nonsteroidal anti-inflammatory drugs (NSAIDS) have also been shown to be ototoxic. This can result in sensorineural hearing loss, dysequilibrium, or both. Some environmental and occupational chemicals have also been shown to affect the auditory system and interact with noise.
Labyrinthitis, also known as vestibular neuritis, is the inflammation of the inner ear. It results in a sensation of the world spinning and also possible hearing loss or ringing in the ears. It can occur as a single attack, a series of attacks, or a persistent condition that diminishes over three to six weeks. It may be associated with nausea, vomiting, and eye nystagmus.
Endolymph is the fluid contained in the membranous labyrinth of the inner ear. The major cation in endolymph is potassium, with the values of sodium and potassium concentration in the endolymph being 0.91 mM and 154 mM, respectively. It is also called Scarpa's fluid, after Antonio Scarpa.
A vestibular schwannoma (VS) is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve. A type of schwannoma, this tumor arises from the Schwann cells responsible for the myelin sheath that helps keep peripheral nerves insulated. Although it is also called an acoustic neuroma, this is a misnomer for two reasons. First, the tumor usually arises from the vestibular division of the vestibulocochlear nerve, rather than the cochlear division. Second, it is derived from the Schwann cells of the associated nerve, rather than the actual neurons (neuromas).
Hyperacusis is a highly debilitating hearing disorder characterized by an increased sensitivity to certain frequencies and volume ranges of sound. A person with severe hyperacusis has difficulty tolerating everyday sounds, which become painful or loud.
Otology is a branch of medicine which studies normal and pathological anatomy and physiology of the ear as well as their diseases, diagnosis and treatment. Otologic surgery generally refers to surgery of the middle ear and mastoid related to chronic otitis media, such as tympanoplasty, or ear drum surgery, ossiculoplasty, or surgery of the hearing bones, and mastoidectomy. Otology also includes surgical treatment of conductive hearing loss, such as stapedectomy surgery for otosclerosis.
Betahistine, sold under the brand name Serc among others, is an anti-vertigo medication. It is commonly prescribed for balance disorders or to alleviate vertigo symptoms, e.g. those associated with Ménière's disease. It was first registered in Europe in 1970 for the treatment of Ménière's disease.
A neurectomy is a type of nerve block involving the severing or removal of a nerve. This surgery is performed in rare cases of severe chronic pain where no other treatments have been successful, and for other conditions such as involuntary twitching and excessive blushing or sweating.
A labyrinthine fistula is an abnormal opening in the inner ear. This can result in leakage of the perilymph into the middle ear. This includes specifically a perilymph fistula (PLF), an abnormal connection between the fluid of the inner ear and the air-filled middle ear. This is caused by a rupture of the round window or oval window ligaments separating the inner and middle ear.
Endolymphatic hydrops is a disorder of the inner ear. It consists of an excessive build-up of the endolymph fluid, which fills the hearing and balance structures of the inner ear. Endolymph fluid, which is partly regulated by the endolymph sac, flows through the inner ear and is critical to the function of all sensory cells in the inner ear. In addition to water, endolymph fluid contains salts such as sodium, potassium, chloride and other electrolytes. If the inner ear is damaged by disease or injury, the volume and composition of the endolymph fluid can change, causing the symptoms of endolymphatic hydrops.
Superior semicircular canal dehiscence syndrome is a set of hearing and balance symptoms, related to a common but rarely diagnosed medical condition of the inner ear, known as superior canal dehiscence. The symptoms are caused by a thinning or complete absence of the part of the temporal bone overlying the superior semicircular canal of the vestibular system. There is evidence that this rare defect, or susceptibility, is congenital. There are also numerous cases of symptoms arising after physical trauma to the head. It was first described in 1998 by Lloyd B. Minor of Johns Hopkins University in Baltimore.
Autoimmune inner ear disease was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing. The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system. AIED is used to describe any disorder in which the inner ear is damaged as a result of an autoimmune response. Some examples of autoimmune disorders that have presented with AIED are Cogan's syndrome, relapsing polychondritis, systemic lupus erythematosus, granulomatosis with polyangiitis, polyarteritis nodosa, Sjogren's syndrome, and Lyme disease.
Vestibular migraine (VM) is vertigo associated with a migraine, either as a symptom of migraine or as a related but neurological disorder; when referred to as a disease unto itself.
Neurotology or neuro-otology is a branch of clinical medicine which studies and treats neurological disorders of the ear. It is a subspecialty of otolaryngology-head and neck surgery, and is closely related to otology, and also draws on the fields of neurology and neurosurgery. Otology generally refers to the treatment of middle ear disease and resultant conductive hearing loss, whereas neurotology refers to treatment of inner ear conditions, or hearing and balance disorders. These specialists also work with audiologists and related sensory specialists.
An endolymphatic sac tumor (ELST) is a very uncommon papillary epithelial neoplasm arising within the endolymphatic sac or endolymphatic duct. This tumor shows a very high association with von Hippel-Lindau syndrome (VHL).
Vestibulopathies are disorders of the inner ear. They may include bilateral vestibulopathy, central vestibulopathy, post traumatic vestibulopathy, peripheral vestibulopathy, recurrent vestibulopathy, visual vestibulopathy, and neurotoxic vestibulopathy, among others.